Objective: To present the study carried out by the Pediatric Surgery Department of Instituto da Criança at the Medical School of Universidade de São Paulo regarding the diagnosis and treatment of children with aortic arch abnormalities and to define the role of complementary exams for diagnosis. Methods: Retrospective study of 22 patients with diagnosis of tracheoesophageal compression treated at Instituto da Criança from 1985 to 2000, analyzing pre- and postoperative clinical data, diagnostic exams and outcome. Results: The most frequent diagnosis was right aberrant innominate artery (10 cases), followed by double aortic arch (7 cases) and right aortic arch (5 cases). Respiratory symptoms (86%) and early manifestation (76% since the neonatal period) were predominant. Nevertheless, most cases (60%) had the definitive diagnosis established only after 1 year of life. The most relevant examination for the diagnosis was the esophagogram. The correction of all the anomalies was carried out through left postero-lateral thoracotomy. There were no surgical complications. The outcome was worse in patients with delayed treatment. All children remained symptomatic for up to 6 months, although they had significant improvement in the postoperative period. Conclusions: The diagnosis of vascular rings should be considered in children with early respiratory symptoms and in the wheezing baby with difficult control. The diagnosis may be established just through the esophagogram. Other image studies add few information and they are unnecessary in most cases. Less severe symptoms may persist for variable periods.